The present invention discloses an antibody specifically binding to a human endothelin receptor and uses thereof, where the antibody can inhibit the functions of the human endothelin receptor. The present invention includes the preparation, cloning, expression, and characterization of the antibody. The present invention can be used to effectively treat pulmonary arterial hypertension, a disease associated with pulmonary arterial hypertension, and a reproductive organ cancer in a human.